Idiopathic pulmonary fibrosis (IPF) is the most common type of pulmonary fibrosis, a condition that causes scarring in the lungs. The condition is called “idiopathic” because the cause is unknown, but research suggests it may be linked to genetic variants, environmental exposure to hazardous substances, history of smoking and GERD. You may initially have no symptoms at all, but as the condition worsens, you may develop a dry cough and shortness of breath with activity.
There’s currently no way to predict how quickly IPF will progress. Scarring develops slowly in some people and rapidly in others. While IPF is a serious condition that has no cure, recent advances in early diagnosis and anti-scarring medications can help relieve symptoms and slow the scarring process in your lungs to help you live longer with better quality of life.
Idiopathic Pulmonary Fibrosis Symptoms
Symptoms of IPF are similar to that of other conditions and tend to come on slowly. At first, you may have no symptoms. You may then develop:
- Dry, hacking cough
- Shortness of breath, or breathlessness with activity
As the condition gets worse, you may notice:
- Unexplained weight loss
- Joint and muscle pain
- Clubbing of the toes and fingertips
In the most serious cases, IPF can result in severe shortness of breath, even at rest. You may also experience an acute exacerbation when your symptoms suddenly get worse for a period of time. These episodes may be brought on by certain triggers such as a viral infection or may come on unexpectedly. During an acute exacerbation, patients may require hospitalization to help get their symptoms under control.
Diagnosing Idiopathic Pulmonary Fibrosis
IPF diagnosis can be challenging because symptoms may be ignored or mistaken for other lung conditions, such as a respiratory infection. But it’s important to get an accurate diagnosis as soon as possible so your healthcare team can design a treatment plan specific to your needs. Diagnosis includes:
Because IPF is a complex disease, a pulmonologist will work closely with radiologists and pathologists to diagnose your condition. Your team will start by ruling out any specific cause for your symptoms. This includes talking to you about:
- Your medical history
- Medications you take
- History of drug use
- Occupational or environmental exposures
- Family history of pulmonary fibrosis or IPF
You will have a physical exam to look for signs of IPF, including clubbing of the fingertips and toes, fluid in your airways and swelling in your arms and legs.
Scarring causes stiffness in your lungs, which in turn makes it difficult to breathe. Doctors measure how much air your lungs can hold, whether your lungs are able to transfer oxygen to the rest of your body, and the effect of exercise on your breathing. Tests include:
- Pulmonary function tests (PFT)
- 6-minute walk test
A high-resolution CT scan is a special type of X-ray that helps doctors visualize the tiny structures of the lungs. Imaging specialists look for usual interstitial pneumonia (UIP) or honeycombing, a type of scarring pattern that is specific to IPF.
Your team may also recommend diagnostic procedures such as bronchoscopy and lung biopsy.
Treatment for Idiopathic Pulmonary Fibrosis
Although IPF has no cure, there are treatments available to help relieve symptoms and slow the scarring that causes damage to your lungs. Your treatment plan may involve a combination of preventive strategies for lifestyle management, supplemental oxygen, pulmonary rehabilitation, medications and procedures. These include:
- Quitting smoking
- Yearly flu and pneumococcal pneumonia vaccines
- Anti-fibrotic medications that help slow the scarring process in your lungs
- Supplemental oxygen therapy
- Treating any other health conditions you may have, including GERD
- Pulmonary rehabilitation to manage acute exacerbation
Lung transplantation can be a life-extending treatment for IPF. There are a number of factors that your doctor will consider when determining if you should be referred for a transplant evaluation, and the procedure is not without risk. Candidates must be in otherwise good physical health with no other life-threatening conditions. They must also be willing to take on the lifelong commitment of caring for their transplanted lungs for the rest of their lives.
Idiopathic pulmonary fibrosis can result in serious, life-threatening complications. These include:
- Acute exacerbation - A sudden and severe worsening of symptoms.
- Respiratory infections - A short-term lung infection is more likely to develop in those with PF.
- Collapsed lung - When air moves from your lung into the chest wall area, causing pressure against your lung. Also called a pneumothorax.
- Pulmonary hypertension - High blood pressure in the arteries leading from your heart to your lungs.
- Acute coronary syndrome - When the blood supply to your heart is blocked, causing damage to the muscle including heart attack and angina.
- Venous thromboembolism - A blood clot that forms in a vein. When the clot breaks free and travels to one or both lungs, it’s called a pulmonary embolism.
- Lung cancer - A growth that grows uncontrollably in the tissues of the lung.
- Respiratory failure - A serious, life-threatening condition that occurs when your lungs can’t get enough oxygen into your blood.
Our Pulmonary Fibrosis Program
Temple’s Pulmonary Fibrosis Program is working to advance understanding of pulmonary fibrosis with the objective of identifying and developing new treatment paths. While there is still no cure for this complex condition, there are now promising treatments that help people live longer with better quality of life. Our team includes experts from every field of research and medicine who are making the commitment to an early and accurate diagnosis. Our top priorities are to stay current with compelling data, house the latest diagnostic technologies, and provide our patients with a comprehensive, long-term approach to treatment.
Temple is proud to offer you:
- A diverse team of specialists who are well-studied in factors that are linked to IPF, including genetics, infectious diseases, gastrointestinal disorders, respiratory conditions and heart disease
- Access to the latest in medication therapy, including anti-fibrotic medications such as nintedanib and pirfenidone
- Involvement in many of the latest clinical trials evaluating novel therapies to treat fibrotic diseases
- A robust pulmonary rehabilitation program to help patients optimize long-term health and overcome the challenge of acute exacerbations
- The highest level of acute care for hospitalization support when needed
- The nation’s #1 lung transplantation program, performing more procedures than any other program, while delivering the best one-year outcomes of any program in the Philadelphia region.*
- Palliative care with a focus on the quality of life for you and your family
*Data Source: the Scientific Registry of Transplant Recipients (SRTR) accumulated volume from data released from January 2015 - January 2022.
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